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Glycogen storage disease Type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of anα-glucosidase inhibitor

✍ Scribed by H. Gröbe; K. Ullrich


Publisher
Springer
Year
1983
Tongue
English
Weight
265 KB
Volume
140
Category
Article
ISSN
0340-6997

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✦ Synopsis


Seven patients with glycogen disease type I have been treated with nocturnal intragastric feeding combined with frequent daytime feeding. Follow-up shows a striking improvement in their clinical condition including growth rate. Determination of biochemical parameters reveals a significant increase in mean blood glucose levels and a significant decrease of lactate, pyruvate, alanine, uric acid, triglycerides, and SGOT in blood. Additional administration of an a-glucosidase inhibitor in four patients caused a significant increase in blood lactate despite unchanged blood glucose levels.